Case -7

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Case -7
SUPRATECH MICROPATH LABORATORY
RESEARCH INSTITUTE
Dr. BHAVANA MEHTA Consultant pathologist Supratech
micropath laboratory and research institute,
Ahmedabad
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Clinical details

• 38 yrs, male
• Renal transplant-5 yrs back
• On cyclosporin ,Azoran, steroids
• S cr- gradually increased upto 3.0, urine
  protein
• Renal biopsy done

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CASE 1

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(No Transcript)
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Provisional Diagnosis

• Post transplant myeloma cast nephropathy

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Why cast nephropathy in post Tx

• Allograft nephropathy
• ARF
• Drug related cast

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Pathogenesis of myeloma cast nephropathy

• Common in multiple myeloma
• Due to over production and precipitation of light
  chain
• Resistant to degradation due to structural
  changes
• Increased urinary calcium due to hypercalcemia
• Acute renal failure due to other disease (volume
  depletion
• Drug related loop diuretics by increasing
  luminal NaCl
• Radiocontrast media interacting with light chains

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What are myeloma casts?

• Precipitated light chains in distal and
  collecting tubules other filtered
  proteinsTamm-Horsfall mucoprotein which a
  protein normally secreted by cells of thick
  ascending loop of Henle and constitute matrix of
  all urinary casts
• Disruption of BM of tubule-leakage-cellular
  reaction

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Other disease with myeloma casts

• Acinar cell carcinoma, pancreas
• Waldenstrom macroglobulinemia
• B cell lymphomas
• Thyroid carcinoma

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History retrieved

• H/o basic disease-Myeloma kidney diagnosed in
  2001
• Urine-Bence Johns protein present
• Protein electrophoresis M band present

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Final diagnosis

• Relapse of myeloma cast nephropathy, renal
  graft.
• Donor-sister
• No family history
• Post transplant period-no problem till 5 yrs

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Post Tx myeloma cast nephropathy

• Usually Tx not considered in myeloma pt.
• Becasuse of risk of recurrence relapse
• If in remission for 1 yr, considered for Tx
• Usually recurrence noted within 2 yrs

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Post Tx myeloma cast nephropathy

• One case reported in early transplant period
  associated with thrombotic microangiopathy
• Upto 10 cases of MM and few with AL amyloidosis
  received Tx- more post Tx mortality
• With recent treatments available for MM- Better
  post Transplant period

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Reasons to present this case

• Young Patient to have MM
• No Bone marrow involvement
• Intraabdominal mass-reported as Multiple myeloma
• Late graft dysfunction-5 yrs
• Presently on Thalidomide and doing well patient

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Thank you